Extramammary Paget’s disease of the vulva is a rare type of superficial skin cancer. It is most common in postmenopausal white women. It is an intraepithelial (layer of cells that forms the surface or lining of an organ) condition that can present as white and red scaly areas on the vulva that may be itchy and painful. The diagnosis is made by examination and tissue sampling. Abnormal cells often extend outside the clinically abnormal area, so some studies suggest frozen section at time of surgery, where a pathologist can give a rapid report of small biopsies to say whether the skin is involved with Paget's or not. Other treatments include: topical medication, such as imiquimod (patient-applied cream); radiotherapy; chemotherapy; photodynamic therapy (form of phototherapy using light-sensitive compounds that are exposed selectively to light, whereupon they become toxic to targeted cancerous and other diseased cells); and laser therapy; or a combination of these approaches. The challenges of interventions are to remove or treat disease that may not be visible, without overtreatment. Avoiding the long-term complications of radical surgery, such as pain and scarring, a feeling of mutilation and loss of femininity, is very important to women. Surgery is still the most common treatment, but it is challenging to remove the disease completely, and recurrence is common, leading to repeated operations, and mutilation of the vulva. The aim of this review was to evaluate the benefits and harms of different treatments for Paget's disease of the vulva.

Study characteristics

We searched for randomised controlled trials (trials where treatment is allocate to women in a random manner) and well-designed non-randomised studies that compared different treatments in women aged 18 years or older with biopsy-confirmed Paget's disease of the vulva.

Key resultsand quality of evidence

We searched scientific databases and contacted experts and identified and checked the titles and abstracts of 635 possibly relevant articles and retrieved 31 of these references in full text. However, we found no studies that met our inclusion criteria. We identified a number of non-randomised studies and drafted a detailed narrative of their results, but these studies were of poor quality and were at high risk of bias. Therefore, there is currently no evidence to determine whether any form of treatment is better or worse in terms of prolonging survival, delaying progression or recurrence, improving quality of life or minimising toxicity. The review highlights the need for good-quality studies comparing different interventions for the management of Paget's disease of the vulva. Women and clinicians would value more evidence for guiding surgical and non-surgical management of this disease. In particular, non-invasive medical management would spare women from the adverse effects and consequences of surgery.